What's the treatment for long QT syndrome?
There are two treatment options in individuals with LQTS: arrhythmia prevention, and arrhythmia termination. Arrhythmia suppression involves the use of medications or surgical procedures that attack the underlying cause of the arrhythmias associated with LQTS. Since the cause of arrhythmias in LQTS is after depolarizations, and these after depolarizations are increased in states of adrenergic stimulation, steps can be taken to blunt adrenergic stimulation in these individuals. These
include administration of beta receptor blocking agents and amputation of the cervical sympathetic chain. Arrhythmia termination involves stopping a life-threatening arrhythmia once it has already occurred. The only effective form of arrhythmia termination in individuals with LQTS is placement of an implantable cardioverter-defibrillator (ICD).
Beta blocker medications are effective for about 90% of patients. A small group of patients may also benefit from other drugs, either instead of or in addition to the beta blockers. In patients who do not respond to medication, the insertion of a pacemaker or defibrillator may be effective. Surgical cutting of certain nerves in the neck, called cervico-thoracic sympathectomy, can be effective as well. All patients with the Long QT Syndrome should be treated, including asymptomatic patients and especially children, because sudden death often occurs with the first episode of syncope and it is not possible to predict which patients are vulnerable.
When LQTS causes uncontrolled ventricular fibrillation, you might need an implantable cardioverter defibrillator (ICD). An ICD is a device that sends an electric shock to your heart to restore a normal heartbeat. The device is placed under the skin of your chest or abdomen and is connected to leads, which are passed through your veins to your heart.